Telangiectases in scleroderma: a potential clinical marker of pulmonary arterial hypertension - PubMed (original) (raw)

Telangiectases in scleroderma: a potential clinical marker of pulmonary arterial hypertension

Ami A Shah et al. J Rheumatol. 2010 Jan.

Abstract

Objective: Clinical markers are needed to identify scleroderma patients at risk for pulmonary arterial hypertension (PAH) since early therapy may improve survival. We investigated whether increased numbers of telangiectases in scleroderma associate with measures of pulmonary vascular disease.

Methods: One hundred forty-seven consecutive adult patients with scleroderma were enrolled in this cross-sectional study and scored for the presence of matted telangiectases on 11 body areas. Per body area, telangiectases were scored as 0 if none were present, 1 if there were fewer than 10 telangiectases, and 2 if 10 or more telangiectases were counted. Linear regression analysis was performed to assess the association between right ventricular systolic pressure (RVSP) and telangiectasia score, adjusted for age, race, smoking status, scleroderma subtype, disease duration, and autoantibody status. Logistic regression analysis was performed with PAH by right-heart catheterization (RHC) as the dependent variable.

Results: The mean telangiectasia score was 6.0 (SD 4.5, range 0-20). RVSP and telangiectasia score were positively correlated (r = 0.271, p = 0.001). The mean RVSP increased by 10.9 mm Hg for every 10-point increase in telangiectasia score (95% CI 3.6-18.3 mm Hg, p = 0.004), adjusted for potential confounders. The adjusted relative odds of PAH by RHC were 12.4 for patients with a 10-point increase in telangiectasia score (95% CI 1.78-85.9, p = 0.01).

Conclusion: Increased numbers of telangiectases strongly associate with the presence of pulmonary vascular disease. Telangiectases may be a clinical marker of more widespread aberrant microvascular disease in scleroderma.

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Figures

Figure 1

Figure 1

Two different types of telangiectasia: (A) matted; (B) stellate.

Figure 2

Figure 2

The mean telangiectasia score increased by 2.5 for every 10-year increase in scleroderma disease duration (p < 0.001).

Figure 3

Figure 3

The mean RVSP increased by 10.8 mm Hg for every 10-point increase in telangiectasia score (p = 0.001).

Figure 4

Figure 4

Total telangiectasia score by pulmonary arterial hypertension (PAH) status. PAH is defined as either an estimated right ventricular systolic pressure (RVSP) ≥ 45 mm Hg or right-heart catheterization–confirmed PAH.

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